2.3. Observation indicators

As all the 9 children were nonlocal patients, they were discharged from the hospital after their symptoms were under control, and subsequently treated mainly via remote guidance. Telephone follow-up checks were conducted every week. Data regarding relevant indicators such as the amount of milk fed, stool, edema, and gastrointestinal reactions were obtained and recorded. In addition, physical development (height, weight), timely review of routine blood test, blood chemistry (including liver and kidney function, electrolytes, levels of blood protein), abdominal B ultrasound, and echocardiography were regularly monitored. Based on these indicators, the nutritional therapy was periodically adjusted as required via remote guidance. The time interval of adjusting the protocol depended on tolerance and status of the children, and the protocol was usually adjusted 1 to 2 times/wk. Because the level of medical care differed for different patients and the parents of some of the children had insufficient knowledge of the disease, the parents tended to perceive improved symptoms as a sign of full recovery, as a result of which it was difficult to follow-up.

Start and endpoints of observation: the time the patients started receiving clinical nutritional therapy was considered the start point; the time at which no clinical interventions, including the use of laxative drugs, blood transfusions, intravenous infusion of human albumin (ALB), and parenteral nutrition, were used was considered as the end point. The end point included management of symptoms, including controlled diarrhea with normal stool (1–2 times/d, formed soft stool) and normal results of routine blood test and blood biochemistry 3 times consecutively.